The Mechanism of Prune Belly Syndrome Development: A Sonographic Sequential Assessment

Abstract

Prune belly syndrome is a rare disorder characterized by the absence of anterior abdominal wall muscles, bilateral cryptorchidism, and urinary tract malformations. The aim of this case study was to illustrate the developmental sequelae of prune belly syndrome, acquired through a series of antenatal sonography. A 20-year-old woman visited a diagnostic medical sonography center for an obstetrical sonogram. Her fetus was diagnosed with megacystis at 15 weeks’ gestation. The mother returned for repeated appointment and to observe fetal changes during the development of prune belly syndrome. First, the megacystis, hydroureter, and hydronephrosis were noted followed by hazy ascitic and amniotic fluid. The hemodynamics were altered in the umbilical artery, middle cerebral artery, and ductus venosus. Subsequently, the pressure was neutralized, but it was observed after birth that the newborn had cryptorchidism, a distended abdomen with wrinkled wall, and absent right kidney. In conclusion, prune belly syndrome could be caused by megacystis due to bladder outlet obstruction in otherwise normal karyotype fetuses. Megacystis leads to hydroureter, hydronephrosis, and abdominal distention. The persistent abdominal distention gives rise to the underdevelopment of anterior abdominal wall muscles and cryptorchidism.

The challenging diagnosis and management of the prune belly syndrome: A case report

“…Severe obstructive uropathy causes distention of the bladder and urinary ascites, which lead to degeneration of the muscles of the abdominal wall and failure of testicular descent. While the impaired elimination of urine from the bladder leads to oligoamnios and pulmonary hypoplasia, 10 an antenatal fetal ultrasound showed oligohydramnios, posteriorurethral valves, or megacystis-megaureter syndrome. While at birth, clinical examination may reveal a newborn with respiratory distress due to pulmonary hypoplasia.…”